Lymphangiomas are rare benign congenital tumours, involving both the head and the neck and causing obstructing symptoms in the upper airways as well as aesthetic anomalies. In recent years, sclerosing therapy with OK-432 has become the treatment of choice in the management of these lymphatic malformations. Nonetheless, surgery still seems to be the therapy advocated for resolution of symptoms. Herein, three cases of lymphangiomas involving the head and the neck are described and a review of the English scientific literature is outlined

Grasso, DL

Pelizzo, G

Schleef, J

Zocconi, E

PubMed

D.L. Grasso

G. Pelizzo

E. Zocconi

J. Schleef

AIR Universita degli studi di Milano

17
Pediatrics
Lymphangiomas of the head and neck in children
I linfangiomi della testa e del collo in età pediatrica
D.L. Grasso, G. PeLizzo1, e. zocconi, J. schLeef1
enT Department, 1 Pediatric surgery Department, irccs “Burlo Garofolo” children’s hospital, Trieste, italy
Summary
Lymphangiomas are rare benign congenital tumours, involving both the head and the neck and causing obstructing symptoms 
in the upper airways as well as aesthetic anomalies. In recent years, sclerosing therapy with OK-432 has become the treatment 
of choice in the management of these lymphatic malformations. Nonetheless, surgery still seems to be the therapy advocated 
for resolution of symptoms. Herein, three cases of lymphangiomas involving the head and the neck are described and a review 
of the English scientific literature is outlined.
Key wOrdS: Head and neck • Lymphangioma • Children • diagnosis • Treatment
rIaSSuNTO
I linfangiomi sono neoformazioni congenite rare e benigne. Essi interessano sia la testa che il collo causando sintomi ostrut-
tivi delle alte vie aeree ed anomalie estetiche. Negli anni recenti, la terapia sclerosante con OK-432 è divenuto il trattamento 
di elezione di queste malformazioni linfatiche. Ciononostante, la chirurgia sembra essere ancora la terapia richiesta per la 
risoluzione della sintomatologia. Gli Autori riportano tre casi di linfangioma interessante il distretto cervico-cefalico ed una 
revisione della letteratura sull’argomento.
ParOLe CHIave: Testa e collo • Linfangiomi • Età pediatrica • Diagnosi • Terapia
Acta Otorhinolaryngol Ital 2008;28:17-20
Introduction
Lymphangiomas are rare congenital benign lesions occur-
ring mainly in the head, neck and oral cavity. They consist in 
localized centres of abnormal development of the lymphatic 
system. Three theories have been proposed to explain the 
origin of this abnormality. The first suggests that a blockage 
or arrest of normal growth of the primitive lymph channels 
occurs during embryogenesis, the second that the primitive 
lymphatic sac does not reach the venous system, while the 
third advances the hypothesis that, during embryogenesis, 
lymphatic tissue lays in the wrong area 1. Lymphangiomas 
are classified as microcystic (capillary lymphangiomas), 
macrocystic (cavernous lymphangiomas) and cystic hygro-
mas according to the size of the lymphatic cavities incor-
porated 2. Bill and Summer, in 1965, presented the concept 
that cystic hygromas and lymphangiomas are variations of 
a single entity and that its classification is determined by 
its location in the head and neck 3. a commonly used clas-
sification classifies these lesions into capillary lymphangi-
oma or lymphangioma simplex, cavernous lymphangioma, 
and cystic lymphangioma or cystic hygroma 4. when a 
lymphangioma is confined to fairly dense tissue, such as 
the tongue, it presents as a cavernous lymphangioma, but 
when it develops in the relatively loose fascia of the neck, a 
cystic lesion occurs. These three types are frequently found 
together in a same patient, depending on the severity of the 
disease. Cystic hygromas, however, account for approxi-
mately 90% of the lymphangiomas in the head and neck 
region. Other common sites, outside the head and neck, in-
clude the axilla, shoulder, chest wall, mediastinum, abdom-
inal wall, and thigh 5. Surgical resection still remains the 
best treatment for lymphangiomas; other treatment options, 
such as sclerotherapy have been proposed as an alternative 
to reduce the impact and complications of surgery. various 
products, such as sodium morrhuate, dextrose, tetracycline, 
doxycycline, bleomycin, ethibloc and OK-432, have been 
used as sclerotherapeutic agents. apart from OK-432, the 
other agents were reported to cause perilesional fibrosis and 
thus to complicate eventual surgical excision 6 7. The present 
report focuses on 3 cases of lymphangiomas of the head 
and neck in children, the therapeutic options adopted and a 
review of the English scientific literature.
Case report 1
Prenatal diagnosis of neck mass
at birth, presence of cystic lymphangioma involving both 
sides of the neck and extending to the floor of the mouth. 
Macroglossia. Sclerosing therapy was started from the first 
month of life with ethibloc® (Ethicon GmbH, Hamburg-
Norderstedt, Germany) medium injection amount of 2 ml 
(alcoholic solution of zein), but results were not successful. 
Sclerosing therapy with OK-432 was performed. Concentra-
ACTA oTorHiNolAryNGoloGiCA iTAliCA 2008;28:17-20
D.l. Grasso et al.
18
tion of oK-432 was 0.01 mg/dl. After fluid aspiration from 
the lesion, the same amount of OK-432 was introduced, up 
to a maximum of 20 ml. injections were performed, with 
ultrasonography (US) guidance, under general anaesthesia 
and following informed consent from the parents.
At 18 months old, during sclerosing therapy with oK-432, 
respiratory distress appeared. Tracheotomy was performed. 
after two months, submandibular and bilateral functional 
neck dissection was performed. Tracheotomy was removed 
after one year. Today, macroglossia and prognathism are 
present. No recurrence of disease in the previous area has 
been found. Lymphangiomatosis, at the tongue base, is still 
present.
Case report 2
Prenatal diagnosis of left submandibular mass
Tongue protrusion and mild face asymmetry were present. 
extrinsic tracheal compression was revealed by computed 
tomography (CT), with no dyspnoea. Sclerosing therapy 
was started from the first month of life with oK-432. Con-
centration of oK-432 is 0.01 mg/dl. After fluid aspiration 
from the lesion, the same amount of OK-432 was intro-
duced, up to a maximum of 20 ml. injections were per-
formed with uS guidance under general anaesthesia and 
with informed consent from the parents. at 6 months of life, 
rapid enlargement of the tongue occurred which required 
nasogastric feeding. respiratory distress required tracheo-
tomy. Submandibular neck dissection was performed. mild 
facial palsy appeared after surgery. At 18 months of life, no 
resolution of tongue enlargement has been obtained. Surgi-
cal reduction of the tongue was performed. Improvement 
in general conditions was observed. Tracheotomy was re-
moved. Today, only mild dynamic facial palsy is present. 
No feeding problems. Speech therapy is followed by the 
patient as the only treatment.
Case report 3
No prenatal diagnosis of neck mass. at birth, a mass was 
present in right neck region. In the following weeks, the 
neoplasm became enlarged. mrI revealed the presence of 
a lesion with inner segmentations in the right neck which 
dislocated the carotid artery and the right internal jugular 
vein. a neck dissection was performed with removal of a 
large multi cystic mass. Histopathology revealed a multi 
cystic hygroma. Three months later, relapse of disease with 
a single cystic lesion. Sclero-therapy was performed with 
OK-432. Concentration of OK-432 was 0.01 mg/dl. after 
aspiration of fluid from the lesion, the same amount of oK-
432 was introduced (maximum 20 ml). injections were per-
formed, under uS guidance and general anaesthesia with 
informed consent from parents. at present, no relapse or 
disease is present.
Discussion
Lymphangiomatous lesions are rare congenital malforma-
tions of the lymphatic system that occur throughout the 
body with greater frequency in the cervicofacial area. Cystic 
lymphangiomas have been reported, in the literature, to be 
present in up to 67% of all cystic lymphangiomas 8. Intro-
duction of prenatal uS allows detection of disease in utero, 
in approximately the same percentage 9. However, almost 
all are detected before the age of two years. Histologically, 
these lesions are composed of dilated lymphatic channels 
with one or two endothelial layers, with or without an ad-
ventitial layer. These dilated lymphatics can vary in size, 
depending upon the location and surrounding tissues and is 
the basis for classification. Cavernous lymphangiomas are 
found in areas, such as the tongue and floor of the mouth. 
Cystic hygromas, on the other hand, arise from lymphatic 
tissue in areas where expansion can occur and large multi-
Fig. 1. Prenatal ultrasonography showing macroglossia and neck 
involvement in patient n. 1.
Fig. 2. CT scan showing the left submandibular mass in patient 
n. 2.
Fig. 3. Macroglossia of patient n. 2.
Lymphangiomas of the head and neck in children
19
loculated cystic spaces can develop. depending upon cystic 
space size, they are classified as: macrocystic, microcystic 
and mixed 10.
The incidence of lymphangiomas has been reported to range 
from 1.2 to 2.8 per 1000 newborns 11. The most prominent 
sign or symptom of all lymphangiomas is the presence of a 
mass. The mass may be small and unnoticed at birth only 
to present later with an upper respiratory tract disorder or 
incidental trauma at the site. most lesions, however, are rec-
ognized early on account of their size and associated symp-
toms of respiratory obstruction and problems with feeding, 
which are the second and third most common presenting 
symptoms. Difficulty in swallowing results from lym-
phangiomas extending to involve the oral cavity, orophar-
ynx, and/or the hypopharynx. Isolated tongue involvement 
can lead to macroglossia with dysphagia and airway ob-
struction. airway and swallowing problems may persist 
after surgery in the neck on account of mucosal oedema, 
enlargement of internal lymphangiomas, and loss of neural 
innervation to the pharynx or tongue. In adult patients, neo-
plasm can switch to squamous cell carcinoma 12.
Diagnosis is not difficult in most cases. The neoplasms are 
usually characterized by the presence of a soft, compress-
ible, loculated and ill-defined mass, which is usually located 
in the posterior cervical triangle. The lesions are not attached 
to the skin or movable across deeper tissues, and readily 
transilluminate 13. The anterior triangle of the neck has been 
indicated as the most common site 14. Cystic hygroma may 
be localized in the parotid area, and is the second most com-
mon congenital mass of the parotid area 13 15.
US, CT and Mri can be used to define the relationship of 
the lesion with the neighbouring structures and to help plan 
surgical strategies 10. uS, which provides size and extension 
of the lesion, should be performed routinely. The clinical 
course of the pathology varies from a spontaneously re-
gressing cyst to an aggressively invasive lesion. Spontane-
ous or traumatic haemorrhage of the cysts is the most com-
mon complication of the lesion 16.
although various methods have been described, surgical 
removal of the neoplasm still appears to be the best choice 
in the treatment of lymphangiomas. Nevertheless, in the 
neck, the close relationship of the tumour to vital structures 
requires softer approaches in order to avoid fatal conse-
quences. On the other hand, total removal of the mass is 
necessary to eliminate the risk of recurrence 17. To this end, 
many authors have described various techniques, including 
serial aspiration, radiotherapy, sclerosing therapy with ster-
oids, alcohol, bleomycin sulphate, tetracycline, and, more 
recently, OK-432 7. The main advantage of OK-432 with 
respect to other sclerosing agents is the absence of perile-
sional fibrosis, and intralesional injection of oK-432 has 
been proposed as the first-line treatment for lymphangioma, 
for the past decade 18. In a recent retrospective study, Oka-
zaki et al. recommended oK-432 injection therapy alone, 
for single cystic and macrocystic types, and surgical exci-
sion after pretreatment with OK-432, for microcystic and 
cavernous types 19.
In our series, not all patients showed resolution of symp-
toms following sclerosing therapy and, indeed, required 
surgical removal. Non-surgical procedures could be pro-
posed in unresectable and recurrent tumours. Classification 
of lymphangioma colli has been described by Schuster et 
al. in order to provide predictive values of post-treatment 
results and occurrence of complications. They identified 
4 types, depending upon volume of the lymphangioma 
itself, as assessed by prenatal uS or clinically. Type I 
included tumours which had no, or only a minimal, ef-
fect on the contour of the neck; type II lymphangiomas 
are smaller than a line drawn at the lateral border of the 
head; type III tumours exceed this line; in type Iv, the 
lymphangioma extends beyond the midline of the body 20. 
Hygromas involving multiple anatomic sites also recurred 
more frequently than lesions confined to a single location. 
Some authors have described cystic hygromas with an 
infrahyoid or suprahyoid location 21. These studies show 
an increased recurrence rate, morbidity, and complication 
rate in those lesions presenting in the suprahyoid region. 
while location of the lesion in the cervicofacial area was 
an important factor for success or failure, the age at which 
surgery was performed did not seem to affect the outcome. 
Complications are more common in lesions involving the 
midline. recurrence rates vary depending upon the com-
plexity of the lesion and the completeness of excision. 
Simple hygromas that are completely excised seldom re-
cur. Complex lesions that are completely excised might 
recur in 10-27%, whereas partially resected lesions may 
recur in 50-100% 22. In our patients, recurrence of disease 
was observed, despite total surgical removal.
Conclusions
Lymphangioma of the head and neck are benign neoplasms 
which are easy to diagnose. Surgical intervention represents 
the treatment of choice. Lesion extension and involvement 
of vital structures can reduce, in some cases, the possibility 
of complete resection. Sclerosing therapy should be consid-
ered for recurrences.Fig. 4. Post-operative image of patient n. 3. 
D.l. Grasso et al.
20
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received: February 12, 2007 - accepted: September 11, 2007


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